primary aldosteronism

原发性醛固酮增多症
  • 文章类型: Journal Article
    作者报告了一例原发性醛固酮增多症(PA),并通过finetenone有效治疗术后醛固酮升高。该患者是一名高血压患者,有30年的高血压病史,5年前患有急性心肌梗死。检测到双侧肾上腺结节伴增生,并证实PA。他的血钾,直接肾素浓度,右肾上腺切除术后醛固酮水平恢复正常。然而,手术后1年,他经历了血钾的减少和醛固酮的增加。盐水输注测试显示醛固酮水平为124.47pg/mL。患者同意使用finenerone治疗。在随访期间,他的醛固酮和钾水平以及血压得到了很好的控制。这种情况强调了尽早筛查继发性高血压的必要性。Finenerone可能对不适合手术的PA患者以及手术后未缓解的患者有效。
    The authors report a case of primary aldosteronism (PA) with postoperative elevation of aldosterone treated effectively by finerenone. The patient was a hypertensive man with a 30-year history of hypertension and sustained an acute myocardial infarction 5 years ago. Bilateral adrenal nodules with hyperplasia were detected and PA was confirmed. His blood potassium, direct renin concentration, and aldosterone level returned to normal after surgery of right adrenalectomy. However, 1 year after surgery, he experienced a decrease in blood potassium and an increase in aldosterone. A saline infusion test revealed an aldosterone level of 124.47 pg/mL. The patient consented to treatment with finerenone. His aldosterone and potassium levels and blood pressure have been controlled well during follow-up. This case highlights the need to screen for secondary hypertension as early as possible. Finerenone may be effective for patients with PA who are not candidates for surgery and those not relieved after surgery.
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  • 文章类型: Case Reports
    背景:低钾性横纹肌溶解症是原发性醛固酮增多症的一种罕见临床表现,使其诊断具有挑战性,特别是当它成为主要表现症状时。在这里,我们介绍1例原发性醛固酮增多症合并低钾性横纹肌溶解症的病例,并进行相关文献复习。
    方法:我们报告了一例54岁的中国男性患者,该患者在过去一年中出现间歇性无力,并因突发性肢体瘫痪2天入院。最终诊断为原发性醛固酮增多症伴有低钾性横纹肌溶解综合征。通过回顾相关的中英文文献,我们注意到自1978年以来只有少数案例发表。排除不相关文献后,我们总结并分析了43例原发性醛固酮增多症伴低钾性横纹肌溶解综合征患者。所有患者恢复良好,随着血钾水平的正常化,大多数人血压恢复正常。一些患者仍然需要药物来控制血压。
    结论:原发性醛固酮增多症很少引起横纹肌溶解;严重低钾血症和横纹肌溶解症的发生应提示鉴别诊断原发性醛固酮增多症。早期发现和治疗对于确定患者预后至关重要。
    BACKGROUND: Hypokalemic rhabdomyolysis is a rare clinical manifestation of primary aldosteronism, making its diagnosis challenging, particularly when it becomes the primary presenting symptom. Herein, we present a case of primary aldosteronism with hypokalemic rhabdomyolysis and conduct a related literature review.
    METHODS: We report the case of a 54-year-old Chinese male patient who presented with intermittent weakness over the past year and was admitted with sudden limb paralysis for 2 days. The final diagnosis was primary aldosteronism accompanied by hypokalemic rhabdomyolysis syndrome. By reviewing the related Chinese and English literature, we noticed that only a few cases were published since 1978. After excluding irrelevant literatures, we summarized and analyzed 43 patients of with primary aldosteronism accompanied by hypokalemic rhabdomyolysis syndrome. All patients showed good recovery, with normalized blood potassium levels, and a majority achieved normalized blood pressure. Some patients still required medication for blood pressure control.
    CONCLUSIONS: Primary aldosteronism rarely causes rhabdomyolysis; the occurrence of severe hypokalemia and rhabdomyolysis should prompt consideration of primary aldosteronism in the differential diagnosis. Early detection and treatment are crucial for determining patient prognosis.
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  • 文章类型: Case Reports
    原发性醛固酮增多症伴3型自身免疫性多内分泌综合征是一种罕见的高内分泌和低内分泌腺体功能的组合。包括手术和替代疗法在内的综合治疗可能是一种有效的策略。
    原发性醛固酮增多症(PA)是源于激素的继发性高血压的常见原因。3型自身免疫性多内分泌综合征(APS-3)的特征是同时或随后发生自身免疫介导的内分泌腺损害,除了艾迪生病.在这里,我们报告了一例极为罕见的病例,即一名63岁的PA和APS-3患者最初患有高血压(HT)。该患者的APS-3主要表现为1型糖尿病(T1DM)和桥本甲状腺炎。她接受了肾上腺腺瘤切除术,组织病理学诊断为肾上腺皮质腺瘤。手术后,该患者的HT立即逆转,血清钾浓度恢复正常。然后,该患者接受胰岛素和左甲状腺素钠片(L-T4)替代治疗.
    UNASSIGNED: Primary hyperaldosteronism with type 3 autoimmune polyendocrine syndrome was a rare combination of both hyper- and hypoendocrine gland function. Comprehensive treatment including surgery and replacement therapy might be an effective strategy.
    UNASSIGNED: Primary aldosteronism (PA) is a common cause of secondary hypertension originating from hormones. Type 3 autoimmune polyendocrine syndrome (APS-3) is characterized by the simultaneous or subsequent occurrence of autoimmune-mediated endocrine gland damage, except for Addison disease. Here we reported an extremely rare case of a 63-year-old woman with PA and APS-3 who initially presented with hypertension (HT). The APS-3 of this patient mainly exhibited type 1 diabetes mellitus (T1DM) and Hashimoto\'s thyroiditis. She underwent the adrenal adenoma resection with a histopathologic diagnosis of adrenal cortical adenoma. After surgery, the HT of this patient was immediately reversed, and the concentration of serum potassium went back to normal. Then, this patient was administered with replacement therapy of insulin and levothyroxine sodium tablets (L-T4).
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  • 文章类型: Case Reports
    原发性醛固酮增多症(PA),通常是由于醛固酮瘤,通常会导致继发性高血压,通常需要手术。我们介绍了一个患有长期高血压的老人,并发脑出血和心肌梗死。增强CT成像可识别右侧醛固酮瘤和左侧肾上腺丰满。结合直立仰卧位醛固酮比率,卡托普利挑战测试,双侧肾上腺静脉取样,和CYP11B1/CYP11B2融合基因检测,确诊为PA。尽管这名患者没有手术干预,药物治疗有效地管理高血压和增强心脏功能,从而强调了醛固酮拮抗剂在诊断为PA的非手术候选者中的有利利用。
    Primary aldosteronism (PA), often due to aldosteronoma, commonly causes secondary hypertension and typically requires surgery. We present a case of an elderly man with longstanding hypertension, complicated by cerebral hemorrhage and myocardial infarction. Enhanced CT imaging identified a right-sided aldosteronoma and left adrenal gland fullness. Combined with upright supine aldosterone ratio, captopril challenge test, bilateral adrenal venous sampling, and CYP11B1/CYP11B2 fusion gene testing, the diagnosis of PA was confirmed. Despite the absence of surgical intervention in this patient, pharmacotherapy effectively managed hypertension and enhanced cardiac function, thereby underscoring the advantageous utilization of aldosterone antagonists in non-surgical candidates diagnosed with PA.
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  • 文章类型: Journal Article
    目的:探讨原发性醛固酮增多症(PA)患者血压(BP)范围内时间(TIR)与复合心血管结局之间的关系。
    方法:2019年1月至2021年12月,在厦门大学附属第一医院招募47例PA患者。在首次诊断PA期间,对所有患者进行了24小时动态血压监测(ABPM)和心血管预后评估。
    结果:患者的平均年龄为48.8±11.4岁。与无复合心血管结局的PA相比,夜间收缩压血压TIR[31.2%(6.2%,81.2%)与11.5%(0.0%,29.7%),p=0.02]和定义的每日剂量(DDDs)的抗高血压药物[2.0(1.0,2.8)与1.0(1.0,2.0),P=0.03]在具有复合心血管结局的PA患者中更低,而更高的葡萄糖(5.0±1.0mmol/Lvs.5.9±1.5mmol/L),并且在具有复合心血管结局的PA患者中,饮酒史的患病率更高。年龄没有差异,性别,BMI,吸烟,高血压的持续时间,脂质水平,醛固酮增多症,临床血压,24小时平均血压,白天或夜间血压,夜间SBP或DBP下降的百分比,24小时BPTIR,白天BPTIR,或夜间DBPTIR两组。在调整混杂因素后,在多元logistic回归分析中,夜间收缩期BPTIR与复合心血管结局显著相关(校正后OR=0.92[95%CI0.86,0.99]).
    结论:PA患者夜间收缩压TIR与复合心血管结局显著相关。
    OBJECTIVE: To investigate the association between blood pressure (BP) time in range (TIR) and composite cardiovascular outcomes in patients with primary aldosteronism (PA).
    METHODS: Between January 2019 and December 2021, 47 patients with PA were recruited from the First Affiliated Hospital of Xiamen University. Twenty-four-hour ambulatory BP monitoring (ABPM) and cardiovascular outcomes were assessed in all patients during the first diagnosis of PA.
    RESULTS: The mean age of the patients was 48.8 ± 11.4 years. Compared to PA without composite cardiovascular outcomes, the nighttime systolic BP TIR [31.2% (6.2%, 81.2%) vs. 11.5% (0.0%, 29.7%), p = 0.02] and defined daily dose (DDDs) of antihypertensive medication [2.0 (1.0, 2.8) vs. 1.0 (1.0, 2.0), p = 0.03] were lower in PA patients with composite cardiovascular outcomes, while higher glucose (5.0 ± 1.0 mmol/L vs. 5.9 ± 1.5 mmol/L) and prevalence of a history of alcohol intake was higher in PA patients with composite cardiovascular outcomes. There were no differences in age, sex, BMI, smoking, duration of hypertension, lipid levels, aldosteronism, clinic BP, 24-hour mean BP, daytime or nighttime BP, percentage of nocturnal SBP or DBP decline, 24-hour BP TIR, daytime BP TIR, or nighttime DBP TIR between the two groups. After adjusting for confounding factors, nighttime systolic BP TIR was significantly associated with composite cardiovascular outcomes (adjusted OR = 0.92 [95% CI 0.86, 0.99]) in multiple logistic regression analysis.
    CONCLUSIONS: Nighttime systolic BP TIR was significantly associated with composite cardiovascular outcomes in patients with PA.
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  • 文章类型: Case Reports
    目的:对于原发性醛固酮增多症(PA)的亚型,建议进行肾上腺静脉采样(AVS)。然而,在PA的情况下,并发亚临床库欣综合征(SCS)有可能混淆AVS结果。Pentixafor,CXC趋化因子受体4型特异性配体,已被报道为评估肾上腺腺瘤功能性质的有希望的标志物。本研究旨在探讨正电子发射断层扫描-计算机断层扫描(68Ga-PentixaforPET/CT)在PA加SCS患者定位诊断中的临床价值。
    方法:两名确诊为PA加SCS的患者接受了AVS和68Ga-PentixaforPET/CT。
    结果:AVS结果显示两名患者均无侧化,而68Ga-PentixaforPET/CT显示单侧肾上腺结节,对68Ga-Pentixafor的摄取增加。根据68Ga-PentixaforPET/CT的结果进行单侧肾上腺切除术。随后,在两种情况下,自主醛固酮和皮质醇分泌均实现了完全的生化缓解。
    结论:68Ga-PentixaforPET/CT在PA加SCS患者中显示出潜在的醛固酮和皮质醇共分泌肾上腺腺瘤的定位。
    OBJECTIVE: Adrenal venous sampling (AVS) is recommended for subtyping primary aldosteronism (PA). However, in cases of PA, concurrent subclinical Cushing\'s syndrome (SCS) has the potential to confound AVS results. Pentixafor, a CXC chemokine receptor type 4-specific ligand, has been reported as a promising marker to evaluate functional nature of adrenal adenomas. This study aims to investigate the clinical value of Gallium-68 Pentixafor Positron Emission Tomography-Computed Tomography (68Ga-Pentixafor PET/CT) in the localization diagnosis of patients with PA plus SCS.
    METHODS: Two patients with a confirmed diagnosis of PA plus SCS underwent AVS and 68Ga-Pentixafor PET/CT.
    RESULTS: AVS results revealed no lateralization for both patients while 68Ga-Pentixafor PET/CT showed a unilateral adrenal nodule with increased uptake of 68Ga-Pentixafor. Unilateral adrenalectomy was performed based on the results of 68Ga-Pentixafor PET/CT. Subsequently, complete biochemical remission of autonomous aldosterone and cortisol secretion were achieved in both cases.
    CONCLUSIONS: 68Ga-Pentixafor PET/CT shows promising potential for the localization of aldosterone and cortisol co-secreting adrenal adenoma in patients with PA plus SCS.
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  • 文章类型: Case Reports
    原发性醛固酮增多症(PA)是一种以高血压为特征的临床综合征,抑制血浆肾素活性(PRA),血浆醛固酮浓度升高(PAC),和自发性低钾血症.
    我们介绍了一名37岁血压正常的低钾血症女性,血浆醛固酮水平高,抑制肾素。患者接受依普利酮和氯化钾补充剂治疗。计算机断层扫描(CT)扫描的进一步检查显示左肾上腺肿块。腹腔镜肾上腺切除术导致肾上腺腺瘤的诊断。
    原发性醛固酮增多症应该是表现为严重低钾血症的正常血压患者的鉴别诊断之一。
    UNASSIGNED: Primary aldosteronism (PA) is a clinical syndrome characterized by hypertension, suppressed plasma renin activity (PRA), elevated plasma aldosterone concentration (PAC), and spontaneous hypokalemia.
    UNASSIGNED: We present a 37-year-old normotensive female with hypokalemia, high plasma aldosterone level, and suppressed renin. The patient was treated with eplerenone and potassium chloride supplement. Further investigation with a computed tomography (CT) scan revealed a mass in the left adrenal. Laparoscopic adrenalectomy led to the diagnosis of adrenal adenoma.
    UNASSIGNED: Primary aldosteronism should be among the differential diagnoses in normotensive patients presenting with severe hypokalemia.
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  • 文章类型: Case Reports
    原发性醛固酮增多症,继发性高血压最常见的可治愈形式,与原发性原发性高血压相比,与高血压相关的器官损伤和心血管并发症更大。作者提出了一个案例,涉及一名41岁的黑人男性因左偏瘫和视力模糊持续一小时而被送往急诊科,伴有明显升高的血压(220/140mmHg)。那时病人已经无症状了,and,除了吸烟和偶尔使用大麻的历史,缺乏明显的医疗合并症。进一步的调查显示右急性出血性中风,双侧4级高血压视网膜病变,慢性肾病与终末期肾病,低钾血症,醛固酮/肾素比率升高。腹部CT扫描显示双侧肾上腺增生。该患者被诊断为原发性醛固酮增多症,伴有广泛的高血压介导的器官损害。该病例突出了未确诊的原发性醛固酮增多症诱发的继发性高血压所造成的重大危害,强调及时诊断和干预以防止器官损伤的重要性。
    Primary aldosteronism, the most common curable form of secondary hypertension, is associated with greater hypertension-related organ damage and cardiovascular complications compared to primary essential hypertension. The authors present a case involving a 41-year-old Black male admitted to the emergency department with left hemiparesis and blurred vision persisting for one hour, accompanied by markedly elevated blood pressure (220/140 mmHg). The patient was asymptomatic by then, and, aside from a history of tobacco smoking and occasional cannabis use, lacked significant medical comorbidities. Further investigations revealed a right acute hemorrhagic stroke, bilateral grade 4 hypertensive retinopathy, chronic kidney disease with end-stage renal disease, hypokalemia, and an elevated aldosterone/renin ratio. An abdominal CT scan showed bilateral adrenal hyperplasia. The patient was diagnosed with primary aldosteronism with extensive hypertension-mediated organ damage. This case highlights the significant harm caused by undiagnosed primary aldosteronism-induced secondary hypertension, emphasizing the importance of timely diagnosis and intervention to prevent organ damage.
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  • 文章类型: Case Reports
    肾上腺皮质癌(ACC)是一种罕见的疾病,预后较差。这基本上需要早期诊断,因为手术是治愈的唯一希望。另一方面,原发性醛固酮增多症(PA)是肾上腺醛固酮的过度产生,被认为是继发性高血压和低钾血症的最常见原因之一。它主要是一种良性疾病。伴有PA的ACC极为罕见,这可能导致延迟诊断和临床陷阱。一名56岁的妇女被诊断出患有PA。温和,有症状的PA通过肾上腺静脉取样(AVS)临床诊断为右侧醛固酮分泌腺瘤(APA)伴肾上腺肿瘤.与典型的良性腺瘤相比,肿瘤影像学发现在计算机断层扫描(CT)上的大小和衰减值异常。十二个月后,该肿瘤被证实为皮质醇分泌过多的ACC。切除的ACC标本未明确显示CYP11B1或CYP11B2阳性结果,怀疑类固醇产生混乱。然而,肾上腺皮质癌的患病率和临床特征与类固醇分泌紊乱仍不清楚.类固醇酶免疫染色分析不仅对于肾上腺腺瘤的诊断很重要,而且对于更好地了解产生激素的ACC的临床过程也很重要。
    Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis, which essentially needs an early diagnosis because surgery is the only hope of a cure. On the other hand, primary aldosteronism (PA) is an overproduction of aldosterone from the adrenal glands and is known as one of the most common causes of secondary hypertension and hypokalemia. It is mostly a benign disease. ACC accompanied by PA is extremely rare, which can result in delayed diagnosis and clinical pitfalls. A 56-year-old woman was diagnosed with PA. Mild, symptomatic PA was clinically diagnosed as a right-sided aldosterone-producing adenoma (APA) with adrenal tumor using adrenal vein sampling (AVS). The tumor imaging findings showed abnormalities on computed tomography (CT) in terms of size and attenuation value compared with typical benign adenomas. Twelve months later, the tumor was confirmed to be an ACC with cortisol hypersecretion. The resected ACC specimen did not clearly show positive findings for CYP11B1 or CYP11B2, and disorganized steroid production was suspected. However, the prevalence and clinical characteristics of adrenocortical carcinomas with disorganized steroid production remain unclear. Steroidogenic enzyme immunostaining analysis is important not only for the diagnosis of adrenal adenoma but also for a better understanding of the clinical course of hormone-producing ACC.
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  • 文章类型: Case Reports
    已知肥厚型心肌病(HCM)是最普遍的遗传性心脏病。然而,关于HCM合并继发性高血压的诊断以及随后的系统治疗的报道有限.在这个案例报告中,我们介绍了一个65岁的男性患者,在身体活动期间反复出现胸部不适,伴随难治性高血压。心脏磁共振成像(MRI)和经胸超声心动图(TTE)显示该个体中存在HCM。进一步调查显示低钾血症,醛固酮水平升高,血浆肾素活性降低,醛固酮与肾素的比率超过30。这些发现强烈表明原发性醛固酮增多症(PA)是影响该患者的其他疾病。通过利用全外显子组测序,我们成功地确定了一个疑似致病基因TTN是患者病情的根本原因。HCM的存在伴随着PA引起的继发性高血压,导致左心室明显扩大,尤其是室间隔.虽然某些基因突变可能表明与心肌病发展有潜在联系,他们不能明确地建立HCM和PA之间的直接关联。
    Hypertrophic cardiomyopathy (HCM) is known to be the most prevalent genetic cardiac condition. However, there have been limited reports on the diagnosis of HCM accompanied by secondary hypertension and the subsequent systematic therapy. In this case report, we present the case of a 65-year-old male patient who presented with recurring chest discomfort during physical activity, along with refractory hypertension. Cardiac magnetic resonance imaging (MRI) and transthoracic echocardiogram(TTE) revealed the presence of HCM in this individual. Further investigation revealed hypokalemia, elevated aldosterone levels, decreased plasma renin activity, and an aldosterone-to-renin ratio above 30. These findings strongly indicated primary aldosteronism (PA) as an additional condition affecting this patient. Through the utilization of whole exome sequencing, we successfully identified a suspected pathogenic gene TTN as the underlying cause of the patient\'s condition. The presence of HCM accompanied by secondary hypertension due to PA resulted in significant enlargement of the left ventricle, particularly the ventricular septum. While certain genetic mutations may suggest a potential link to cardiomyopathy development, they cannot definitively establish a direct association between HCM and PA.
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